Immunotherapy of familial hemophagocytic lymphohistiocytosis with antithymocyte globulins: a single-center retrospective report of 38 patients

N Mahlaoui, M Ouachée-Chardin… - …, 2007 - publications.aap.org
OBJECTIVES. Familial hemophagocytic lymphohistiocytosis is a genetically determined
condition that is characterized by unremitting CD8 T lymphocyte and macrophage activation
and leads to death in the absence of therapy. On the basis of the immunologic
pathophysiology of familial hemophagocytic lymphohistiocytosis, we propose a therapy with
a combination of antithymocyte globulins with corticosteroids, cyclosporin A, and intrathecal
injections of methotrexate. METHODS. We retrospectively analyzed the outcome of …